| Entity | t(11;14)(p13;q11)/T-cell leukaemia --> LMO2 - TCRD-A |
| Disease | Childhood T-cell ALL ; found in 5-10% of T-cell ALL. |
| Cytogenetics | A variant translocation t(7;11)(q35;p13) has been described. |
| Abnormal Protein | It was previously believed that LMO2 is activated after chromosomal translocation by association either the T-cell receptor a / T-cell receptor d (14q11) or T-cell receptor b gene (7q35). Chromosome breakpoints occur 25 kb upstream LMO2 gene, in a presumed transcriptional start site, inducing truncation of the promoter/control region and leading to inappropriate Lmo2 level especially in T-cells (abnormal T-cell differentiation). However, it becomes now very likely that removal of a negative regulatory element from the LMO2 locus, rather than juxtaposition to the TCRD enhancer, is the main determinant for LMO2 activation in the majority of t(11;14)(p13;q11) translocations. |
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| Entity | del(11)(p12p13) T-cell leukaemia |
| Disease | Childhood T-cell ALL; found in about 5% of T-cell ALL. |
| Cytogenetics | Cryptic deletion that varies in size. |
| Abnormal Protein | LMO2 is activated through a cryptic intrachromosomal deletion, del(11)(p12p13), in which a negative regulatory element (NRE), situated upstream of the LMO2 gene, is deleted. Removal of this NRE causes activation of the proximal promoter of the LMO2 gene leading to its ectopic expression. |
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| Entity | Germinal center B-cell lymphomas |
| Disease | Diffuse large-B-cell lymphomas, follicular lymphomas, Burkitt lymphomas, less often in other haematological malignancies. |
| Prognosis | LMO2 expression, together with BCL6, FN1, CCND2, SCYA3, and BCL2 expressions, is a predictor of outcome in diffuse large-B-cell lymphoma. |
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| Entity | Prostate cancer |
| Note | Expression of LMO2 is higher in prostate tumours samples than in the normal epithelium. Moreover, overexpression of LMO2 is significantly associated with advanced tumour stage, as well as with the development of distant metastasis. |
| Oncogenesis | LMO2 may play an important role in prostate cancer progression, possibly via repression of E-cadherin expression. |
| | |
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| Human Bex2 interacts with LMO2 and regulates the transcriptional activity of a novel DNA-binding complex. |
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| Different chromosomal breakpoints impact the level of LMO2 expression in T-ALL. |
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| The Lim-only protein LMO2 acts as a positive regulator of erythroid differentiation. |
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| Protein stability and transcription factor complex assembly determined by the SCL-LMO2 interaction. |
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| The significance of LMO2 expression in the progression of prostate cancer. |
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| The oncoprotein LMO2 is expressed in normal germinal-center B cells and in human B-cell lymphomas. |
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| PMID 17038524 |
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| A novel transcript of the LMO2 gene, LMO2-c, is regulated by GATA-1 and PU.1 and encodes an antagonist of LMO2. |
| Wang Q, Zhang M, Wang X, Yuan W, Chen D, Royer-Pokora B, Zhu T |
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| A highly conserved regulatory element controls hematopoietic expression of GATA-2 in zebrafish. |
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